Treatment of gastrointestinal disease in systemic sclerosis (scleroderma)
- Stephanie A Kaye-Barrett, MD
Stephanie A Kaye-Barrett, MD
- Consultant and Honorary Lecturer in Rheumatology
- The Chelsea and Westminster Hospital
- Christopher P Denton, MD
Christopher P Denton, MD
- Professor of Experimental Rheumatology
- Royal Free Hospital, London
Nearly 90 percent of patients with systemic sclerosis (SSc, scleroderma) have some degree of gastrointestinal (GI) involvement [1,2]. The earliest visceral manifestation to be described is generally esophageal disease, which remains the most common source of GI symptoms in SSc, but any part of the GI tract (mouth to anus) may be involved (table 1).
Clinically significant GI dysfunction occurs in approximately 50 percent, with severe involvement (such as malabsorption and intestinal pseudo-obstruction) being observed in less than 10 percent and portending a poor outcome [3,4]. Approximately 70 percent of patients die within three years of the onset of malabsorption, recurrent pseudo-obstruction, or the requirement for hyperalimentation .
The management of GI disease associated with SSc will be discussed here. The remaining issues relating to GI dysfunction, including pathogenesis, pathology, clinical manifestations, and diagnosis, are presented separately. (See "Gastrointestinal manifestations of systemic sclerosis (scleroderma)".)
The general management of gastrointestinal (GI) manifestations is outlined in the table (table 2). The appropriate therapy depends upon the location of involvement and resulting symptoms. It is important to recognize the nuances and subtleties of symptoms, since small differences in presentation may result from entirely different pathogenetic mechanisms. As an example, intermittent diarrhea or constipation is most commonly due to intestinal dysmotility and/or pseudo-obstruction, while persistent diarrhea is likely to be secondary to malabsorption and bacterial overgrowth.
The treatment of oral manifestations of systemic sclerosis is largely supportive. As examples:
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- OROPHARYNGEAL DISEASE
- ESOPHAGEAL DISEASE
- Gastroesophageal reflux
- Barrett’s esophagus
- Fungal infection
- GASTRIC DISEASE
- SMALL INTESTINAL DISEASE
- Pseudo-obstruction and small bowel failure
- Pneumatosis cystoides intestinalis and pneumoperitoneum
- COLONIC AND ANORECTAL DISEASE
- Fecal incontinence
- BILIARY TREE DISEASE
- PANCREATIC DISEASE
- SUMMARY AND RECOMMENDATIONS